4

No Case for Euthanasia

the quality of life of vegetative and locked-in patients

Who hasn’t heard of the famous arrow poison used by some indigenous people of South America? Usually known as curare, it is a beloved plot device of writers of adventures stories and crime thrillers, as it turns its victims into puppets unable to control their own movements, paralysing their muscles, including those required for breathing, until they eventually exhale their last. Silently, inconspicuously. As the famous German explorer and naturalist Alexander von Humboldt remarked during his Latin American expedition, curare kills more stealthily than any other drug, and, as a man of science, Humboldt was not given to exaggeration.

We now know that curare achieves this effect by blocking certain neuromuscular receptors, which pulls the plug, as it were, on any kind of motor activity. Curare victims are still able to perceive their surroundings, to think and to reason, but they cannot move. At all. They are completely paralysed. As horrific as it sounds for the victim, for scientists this effect is highly interesting. Some researchers — including myself — have experimented with self-curarisation. Of course, it must be done in the presence of an anaesthetist to make sure breathing continues. The surprising result is that, as long as there is sufficient trust in the anaesthetists, curarisation is experienced as extreme relaxation. This is because curare paralysis prevents the muscles from communicating fear to the brain.

The curare scandal

In the late 1960s, a news report emerged from the USA that shook the psychological world to its foundations. It appeared that learning behaviour and thinking was not, as had been believed until then, completely unconnected to the control of physiological processes such as the beating of the heart or excretion of stomach juices — meaning that both types of processes were basically the same and so could also be influenced and controlled in the same way.

The source of this revolutionary idea was the experimental psychologist Neal A. Miller, of New York’s Rockefeller University.8 He injected rats with curare to exclude any influence of movement or motor function on their learning. He then systematically rewarded certain changes in their bodily functions. For instance, if their heartrate increased in response to a certain signal, the reward centre of their brain was stimulated electrically, causing a pleasurable sensation. The alleged result was that the animals learned to increase their own heartrate without any external stimulus, purely in the hope of receiving a pleasurable reward. The same appeared to be true of other physiological changes, such as raising blood pressure, increasing blood supply to the kidneys, and modulating brainwave frequency. The rats were even taught to dilate the blood vessels in one ear more than the other, so that one took on a vaguely blue hue, while the other was white. Yes, the rats had different coloured ears — not because they had been dyed or placed with an infrared lamp shining only on one side of their head, but because they had manipulated their blood circulation at will, despite being paralysed with curare.

Not surprisingly, these results attracted a lot of attention. And since the circulatory system and the nervous system are similarly structured in both rats and human beings, the conclusion was that humans could perform the same feats. In other words, it was assumed that humans could control their bodily functions in the same way, without any physical activity being involved in the process and without the need to develop a particular behaviour. The conclusion was that these physical changes could be achieved by pure will, if a person wanted to receive a reward.

It seemed that the borders between medicine and psychology had been broken down. Many research scientists, including myself, began to dream. We believed it would only be a tiny step until unhealthy or pathological physical and mental processes could be corrected using concentration alone. Gastritis? No problem! All you need to do is practise relaxing your stomach and secreting less gastric acid. Anyone could treat their own depression by simply learning consciously to alter the brainwave frequency causing their depressive state. We even dreamed of starving cancer cells to death simply by teaching patients to focus on cutting off the blood supply to their tumours. It seemed as if almost every kind of illness would soon be curable just through learning, without the need for pharmaceuticals. As young researchers, we went on pilgrimages to New York to breathe the intoxicating air of omnipotence itself, and it was suggested that Miller should receive a Nobel Prize.

However, we were soon to be brought back down to earth. Not one single researcher was able to replicate the results of Miller’s curare experiments. When one of Miller’s assistants eventually committed suicide, suspicions began to grow that Miller had manipulated the results, and those suspicions remain to this day. Could it all have been nothing but a great big con? Whatever the truth, Miller’s name was removed from the list of potential Nobel Prize candidates, and he lost his job at Rockefeller University, and our dreams burst like bubbles.

Nonetheless, for me, the episode was important as an impulse for future theories, and I am still proud to have been a student of Miller’s. Ironically, that important intellectual impulse did not bear fruits until I was forced to take my ideas in a different direction. We wanted to establish contact with our locked-in patients, who lie in their beds completely paralysed like curare victims, and Miller’s failure meant we had to develop a different strategy, an alternative to deliberate reward-based learning. After all, why would completely paralysed people become active and communicate with us, when they were no longer able to achieve any effect? If rats didn’t do so, why should human beings, with the ability to reflect rationally on their actions, be any different? I came upon the idea that the answer could be found in the work of a classic behavioural researcher: the legendary Ivan Pavlov.

From reflex to volition

This Russian psychologist discovered that just the ringing of a bell is enough to make dogs salivate if they have been conditioned to associate the sound with the presentation of food, thanks to the repeated occurrence of the auditory stimulus at the same time as the dogs were fed. After a certain number of repetitions, the formerly neutral stimulus alone was enough to trigger salivation in the dogs. Pavlov called this a conditioned reflex. It is important to note that this was a reflex and not an act of volition.

We decided to make use of this mechanism with our locked-in patients, by asking them questions that did not require deliberation or decision-making, but which could be answered spontaneously. Examples of such questions included: ‘Is Paris the capital of France?’ ‘Is Paris the capital of Germany?’ ‘Is up the opposite of down?’ The answers to such questions can be given reflexively as ‘yes’ or a ‘no’, without the need for further consideration. During this process, we used near-infrared spectroscopy to observe what was going on in our patients’ brains. A computer program then used these observations to create a pattern to be used in turn as a basis for comparison for later experiments. In other words, we taught the computer program to recognise the brain activity of patients when they answered ‘yes’ or ‘no’.

The next step was to confront patients with question that they could not answer reflexively. For example: ‘Would you like to see your children?’ And then the crucial question: ‘Do you want to die?’ The computer recognised whether patients answered ‘yes’ or ‘no’, after which we turned the question around, as a control (‘Do you want to live?’ ‘Do you want your children to stop visiting you?’). Communication was successful, and we were able to recognise answers reliably.

Although it did not work all the time, the overall implication was clear: locked-in syndrome does not necessarily entail a total departure from life. Patients could indeed communicate if they were released from their prison in a systematic way. However, this did not address one important issue: that of the will to live, of a satisfying and fulfilled existence. It may well be a welcome change for locked-in patients confined to their curare cage to re-establish contact with the world. But is that enough to make a person happy?

Can it be called a life?

Anyone who tries to imagine what locked-in syndrome must be like for a patient cannot but shudder at the thought of suffering such a gruesome fate. Let’s say you have suffered a stroke and have just been taken to hospital. There you are, lying in a closed room, staring at the ceiling. In the coming weeks and months, that ceiling will become your constant companion. Friends and family come to visit you, but many of them are struggling to cope with the situation, such that you are relieved when their visits are over. You are left alone with your thoughts. You think of the sex that you will never have again, of your mother’s delicious beef roulade that you will never eat again, of that after-work beer you’ll never drink again. All that remains of the real world for you is the hospital room around you: the soulless beeping of machines rather than the joyful wagging of your dog’s tail; the smell of disinfectant rather than the scent of your favourite perfume.

And no doctor tries to ascertain whether you are still aware of anything, as that isn’t normally part of a hospital’s standard bank of tests. The hospital staff in general are not much help to you. They can be divided into four groups: the businesslike ones, who go about their work with indifference; the cynical ignoramuses, who think you are just a malingerer; the rough, inconsiderate ones, who believe you can’t feel anything anymore anyway; and finally, those who are genuinely interested in your fate and whose efforts are, on the one hand, like balm for your soul, but, on the other hand, increase your desire for normal conversation to an unbearable level. In any case, you no longer feel that you are being treated like a fully-fledged member of the human community.

Perhaps the idea passes through your mind — many things pass through your mind at this time, since your mind is the only part of you that remains functional — that all this is a blessing in disguise. After all, you could have suffered the same fate as the Belgian man Rom Houben. It was 23 years until he was diagnosed with locked-in syndrome! For all that time, he was presumed to be comatose and in a persistent vegetative state, unaware of anything or anyone around him. Very few patients who are assumed to be comatose or in a persistent vegetative state, or, in more modern medical parlance, with ‘unresponsive wakefulness syndrome’, are examined as closely as they would be by Steven Laureys in Liège and me and my team in Tübingen. That alone is a scandalous state of affairs. But fortunately, you are spared that fate, because you are attached to an EEG monitor just days after your stroke and are confronted with senseless statements like ‘fingernails grow on trees’ or ‘elephants have wings’, which provoke your logic-loving cerebrum into firing off electronic flashes of indignation.

But you are unable to draw much comfort from the story of Rom Houben. After all, even when your condition has been diagnosed correctly, your life remains bleak, with little stimulation. Not to mention the fact that you have already been told that you will never return to your old state of health. And that you will require constant care for the rest of your life — eternally dependent on the help of others; a burden on all your loved-ones. Happiness is a thing of the past. All that remains for you is agony and despair. Or not?

The meaning of life beyond iPhones and marathons

This or something similar is the way most healthy people imagine the experience of total paralysis. It is inconceivable for them that anyone could experience contentment or happiness in such a situation. But is it really so impossible? And how can we ever find out?

Sociologists and psychologists have developed certain tests to sound out various ‘happiness factors’, as a way of measuring individuals’ level of satisfaction with their day-to-day lives. They include questions such as ‘Do you have a lot of friends?’, ‘Do you enjoy eating out with friends?’, ‘Are there things in life which make you happy?’, ‘Do you like getting up in the morning?’, and ‘Do you enjoy sex?’. The answers are analysed using a points system to provide an evaluation of the test person’s general quality of life. It works in a similar way to an intelligence test and — provided the test person does not lie — has great informative value.

For locked-in patients, however, many such questions lose their meaning, because those patients are no longer able to move. They may be able to indicate whether they have a lot of friends or not, but they are not even able to eat, let alone go out to eat with their friends. Sex is usually also a thing of the past, and even getting up in the morning is an impossibility since they are unable to even sit up without assistance. After a time, their brain stops wanting to move, or at least stops wanting it so much, and questions about movement then no longer have the same significance to locked-in patients as they would for a healthy person.

For this reason, we came up with a new catalogue of questions, which do not focus on movement and which can be answered with equal relevance by both paralysed and able-bodied respondents. The questionnaire includes statements like ‘I have good friends I can rely on’ and ‘On the whole, I am happy with my life’, which can be answered simply in the negative or the affirmative. The answers given showed that both groups have about the same quality of life. In the case of some paralysed patients, the result was significantly higher, although for others it was also significantly lower than for the healthy respondents. This depends on the current stage of their condition.

Patients with amyotrophic lateral sclerosis (ALS) often feel very unhappy in the early stages of their illness, and many are plagued by thoughts of suicide in this period. One reason for this is that the end of their normal life as a mobile human being is still recent and raw; another reason is their fear of what is to come. And this fear has been found to be especially strong in doctors who are diagnosed with ALS. This is presumably because, while doctors may be aware of the way this muscular paralysis leads to the physical decline of the whole body, they never previously considered how to deal with this challenge mentally.

The mind’s potential for coping in this way is huge, which is why many ALS patients achieve an impressive level of life quality as their disease progresses. They do experience a difficult crisis period when their lungs begin to stop functioning — they repeatedly experience feelings of suffocation, and eventually they need to be ventilated mechanically through a tube. This can lead to a resurgence of thoughts of assisted suicide. But once they have passed that hurdle, their quality of life can once again stabilise at a high level. This happens when such patients learn to adapt their priorities to their physical condition and let go of the priorities they had before they became paralysed.

As their condition progresses, the focus of their attention falls increasingly on those they share their lives with and on social interaction, while the pleasures of the able-bodied, such as going out or engaging in sports, begin to pale. The people they are close to, usually family members, become increasingly important, and this is why it is crucial that those people do not break off contact with the patient. People who are totally paralysed may no longer be able to run marathons or even swipe their fingers over an iPhone screen, but life still provides them with enough moments of happiness. They will ‘say’ such things as ‘I feel happy whenever my husband comes in’ or ‘My biggest joy is when my son comes to visit at the weekend’.

It is perfectly possible for a severely paralysed person to lead a worthwhile life once they have mentally accepted their physical condition — and it is very likely that they will do so. Our tests show that most such patients do very well in coming to terms with their situation and achieve a high quality of life on the basis of what is still possible for them. And these people include many who believed categorically when they were still healthy that this would never be possible for them to achieve.

Signals of happiness from the depths of the brain

Nevertheless, we came in for some hefty criticism for our findings on the quality of life of such patients. It was suggested that our patients had only reported having a high quality of life out of a desire not to disappoint us, the research team. The proposed explanation for this was that we and our machines were, so to speak, all they had left in the world and they did not want to jeopardise that. The reality, according to those who spearheaded this psychoanalysis-based criticism, the patients were weary of life and were just concealing the fact — albeit mostly unconsciously, since most fears are fed by the unconscious mind — so as not to lose that final point of contact with their surroundings. In other words: our findings on our patients’ quality of life were worth about as much as a confession extracted under torture from an inmate at Guantanamo. Neither statement originates in free will, but under external pressure.

We did not waste time on considering why a person with an incurable condition should be more likely to lie about their quality of life than a healthy person who had much more to lose by comparison. However, since we doubted that deductions made without ever having communicated seriously with severely paralysed patients could lead to knowledge of their ‘true’ motives, we set about measuring the life quality of our patients in a scientific way.

To do so, we used magnetic-resonance imaging, because that technology allowed us to see changes in blood supply to the deeper regions of the brain. Patients were placed in an MRI scanner and confronted with images (for those who still had use of their eyes) or sequences of sounds (for those who could only hear) intended to provoke positive or negative emotions in them. Members of a control group of healthy volunteers were subjected to the same procedure. Prior to the start of the experiment, members of the locked-in patients’ families were asked whether it was acceptable to subject them to the emotionally charged sequences of images or sounds.

The images and sounds were taken from the International Affective Picture System (IAPS), developed at the University of Florida by our friends Peter Lang and Margaret Bradley. This material is used by psychologists all over the world as a standardised tool for studying and recording the way emotions are triggered, and it includes images that would affect anyone in the world more or less intensely, irrespective of their cultural background. The set contains images of, for example, a pile of human skulls, maimed children, and dismembered human corpses to provoke reactions of fear, horror, and disgust in anyone who sees them. There’s images of naked women (for male subjects) and laughing infants (for female subjects) to provoke feelings of lust or joy. The IAPS consists of hundreds of images, which have been tested on countless numbers of people and have been classified on a scale of one to ten for their emotional effect. For subjects without visual perception, there is a set of corresponding sounds, including the sound of the sea and of children’s laughter, as well as human cries of pain and the screech of a circular saw.9

When we exposed our patients and the group of healthy volunteers of similar ages to the pictures or sounds, we found some remarkable differences in their MRI scans. The locked-in patients reacted more strongly to positive stimuli and less strongly to negative stimuli, and this tendency was particularly noticeable in patients who had been ill for some time and were reliant on artificial ventilation. There was no indication at all that their negative physical condition left them able to react only weakly to their environment. Contrariwise, there was also no sign that their extremely low-stimulus life caused them to be shocked or to over-react, which might have been expected. Rather, that which makes us happy made these highly restricted people even happier, and that which makes us unhappy affected them much less than us. Ultimately, the conclusion must be that their quality of life is higher than ours.

Subjects’ reactions to the IAPS stimuli show up in MRI scans in different areas of their brains, depending on which signalling pathways are activated. For instance, images of happy, friendly faces affect mainly the supramarginal gyrus, a part of the cerebral cortex found in both hemispheres where the parietal, temporal, and occipital lobes meet. When this area is active, it sends blocking signals to the amygdalae and other parts of the neuronal system involved in reacting to danger. Those blocking signals cause us to relax and make a friendly face. This is a very useful mechanism for a communicative species such as human beings, as it helps us reach an emotional consensus. And this appears to be all the more so for human beings who are extremely limited in their ability to communicate, because this lends more significance to each individual communicative signal.

Our patients’ supramarginal gyri reacted significantly more strongly when they were shown images of good-humoured faces than those of the healthy control group, which means our patients’ brains switched more strongly to a state of positive relaxation. They were more ready to put on a friendly face — even though the paralysis of their facial muscles meant they couldn’t show it.

All this raises the question not only of why people who can barely do anything at all should continue to react to their environment, but also why they should have a more-than-averagely positive attitude to life. Shouldn’t we expect their brains to just give up?

What goes around …

To answer this question, we looked more closely at the actual lives of our patients. We found that it was particularly those who were in an environment where they felt well cared for — that is, those with a good relationship with their carers and their families and friends — who felt the most joy for life. To put it another way: locked-in patients who are surrounded by friendly, caring, and empathetic people feel good. Their brains are ultimately mirrors of their environment, just like everybody else’s.

Someone who spends the whole day with malicious people will eventually have a brain that is tuned to maliciousness. Alternately, the brain of someone who spends the whole day with communicative, friendly people will be calibrated for friendly interaction. This is true of every human being, and the effect is all the greater for those who are dependent on the help of others and are able to communicate only very little due to a severe disability.

This stabilisation of, or even increase in, a patient’s quality of life can be observed not only in stroke survivors, but also in those with other conditions that cause the brain to lose control over movement and other important functions. These include ALS, Parkinson’s disease, epilepsy, paraplegia caused by injury, and advanced dementia. Even patients with lingering physical diseases that lead to infirmity, such as AIDS and rheumatism, often display an impressive lust for life — as long as their pain can be controlled — and show no sign of resignation. Except for some forms of cancer that cause brain tumours that disrupt the hormonal balance of the brain to such a degree that the sufferer’s mental state cannot be stabilised, there are virtually no very severe diseases that prevent the sufferer from achieving a high quality of life.

It is not uncommon for people to gain strength precisely through becoming ill to make radical changes to their lives, such as ending a destructive relationship, quitting a frustrating job, or taking that trip of a lifetime they have always dreamt of. Once they have mentally processed their diagnosis and become accustomed to their altered life conditions, many people feel energised and optimistic. We are not talking about a handful of people here, but millions of human beings whose lives often may not seem worth living from the point of view of a healthy person, but who themselves feel their lives are very worthwhile. This insight should always be at the centre of any discussion about living wills, assisted suicide, and euthanasia.

Living wills: enticement to suicide

For many years, living wills (or as the medical profession often terms them: ‘advance healthcare directives’) led a shadowy existence. It was seen as taboo to think about that ‘worst-case scenario’, when decisions would need to be made about how and if we should be treated if serious illness leaves us no longer able to communicate. But those times are over in an increasing number of countries around the world.

Since the corresponding legislation was passed in my country, every citizen has the right to determine in writing ‘whether he consents to or prohibits specific tests of his state of health, treatment or medical interventions not yet directly immanent at the time of determination (living will)’ (Section 1901a of the German Civil Code). The Humanist Association of Germany estimates 12 million people throughout the country have now signed living wills as a result of that legislation. Some of those may not be legally watertight, but the trend is clear: citizens want to decide for themselves in advance whether everything medically possible should be done to keep them alive if serious illness leaves them unable to express their wishes, or if treatment should be stopped and they should be allowed to die. This sounds like progress and self-determination — but in fact it is essentially the opposite.

How can there be talk of a freely taken decision if a living will signed by a healthy 40-year-old man is applied 30 years later only because he has been robbed of his ability to revoke it — due to advanced Alzheimer’s disease or ALS, for example — although he is actually quite satisfied with his life? Indeed, the time between signing the living will and its application need not be so long: under German law, even seriously ill people can usually sign a living will.

Thus, in the knowledge that their illness will eventually lead to locked-in syndrome, many ALS patients sign a living will stating that they do not want to be artificially ventilated. When their breathing becomes progressively difficult, and their condition therefore becomes increasingly distressing, they insist on ending their suffering and urge the medical staff treating them to help them do so.

In Tübingen, we usually refuse such requests. We do not do this out of high-handedness or lack of sensitivity, but because — unlike the patients in question — we know how great the chances are that they can still attain a high quality of life. This is because their brain will adapt to receiving only very few external stimuli, and, by the same token, those stimuli will be experienced as particularly intense and positive, as long as the patient is well cared for. We explain this to our patients in great detail and ask them to place their trust in us and to have a little patience so that they can live to experience this effect themselves. If they agree, we sometimes even tear up the actual living-will document.

Our patients can, of course, rely on us to support their rights and follow their wishes if their love of life should ever be irrevocably lost — even without a formal declaration. Having said that, such a case has never occurred.

Football and skiing to the very end

How wrong-headed a living will can be is illustrated by the case of one of the first patients we managed to teach to communicate with us using his brain, despite being paralysed by ALS. His name was Hans-Peter Salzmann, and, during his career as a judge in Stuttgart, he had gained a reputation as a reliable person who stuck by court decisions once they were made. After receiving the devastating diagnosis of ALS, he put pen to paper and with characteristic meticulousness drew up a living will, which he put away in his desk drawer. The will included his wish not to be artificially ventilated.

A few months later — he was still being cared for at home — he was suddenly unable to breathe. His carer called the emergency doctor, who immediately put Hans-Peter on artificial ventilation. This was against the patient’s wishes, but the doctor knew nothing of the will expressing those wishes, which remained undiscovered in that desk drawer, and Hans-Peter was no longer able to communicate.

After he was admitted to a clinic, he heard about us and our brain–machine interface, with its possibility of enabling almost completely paralysed people to communicate with their environment. Hans-Peter decided to seize this opportunity. However, when communication was successful and he was even able to dictate entire sentences via the BMI, he did not mention a single syllable about his living will.

For eight years, he not only remained alive, but also enjoyed taking an active part in that life. His favourite pastimes in that period included watching football and downhill skiing on the television. He had been an active football player and skier earlier in life, but we had assumed that a totally paralysed person would gradually lose interest in such things, since their irreversible state of inactivity would destroy the associated motion patterns in the brain. However, our judge from Stuttgart remained faithful to his favourite sports.

A smile on a frozen mask

Hans-Peter left us in no doubt that he considered his life worth living. We watched sports shows together and, in the notes he would dictate via the BMI, he would even invite us to parties, where we would use a funnel to pour a glass of wine into his enteral feeding tube. On those occasions, we all had the undeniable impression that his face took on a happy and relaxed expression. Which was surprising, as the paralysis of his face muscles should really have made that impossible. The faces of ALS patients are usually frozen and mask-like, and so, after thinking about it rationally, we concluded that we must have imagined the change in Hans-Peter’s facial expression.

However, we later realised that our first impression was probably correct, after all. Human facial expression also involves muscles that are not subject to motor control in the brain — these also include those muscles involved in causing the hairs on our skin to stand on end when we get goose bumps — and which can therefore still function in totally paralysed patients. Moreover, those muscle movements can sometimes become particularly visible in patients with ALS, precisely because their other, consciously-controlled muscles atrophy and no longer dominate any facial expression.

As well as this activation beyond any motor control, another feature of the involuntary muscles of facial expression is that they are involved not only in expressing emotions, but also in the opposite effect of initiating, or at least reinforcing, emotions in the brain. The principle: smiling outwardly creates stimuli that also make us smile inwardly! It is not for nothing that some behavioural therapists recommend that their depressed patients somehow try to put a smile on their faces, as this will also affect their mental state.

Therefore, we must assume that Hans-Peter not only looked particularly happy after his glass of wine, but that he actually was happy. Later, we saw the same effect with a Peruvian ALS patient, although it was whisky rather than wine that his wife funnelled into his feeding tube.

Apart from this effect, Hans-Peter of course communicated less via his facial expression and more via the brain–machine interface, by activating specific regions of his brain to create impulses that a computer program translated into syllables. As he was still able to see, he was also still able to recognise letters, syllables, and words on a computer screen. If he wanted to select a certain letter, he would have to create a slow brain potential — a technique he had learned and practised. If he was successful, a series of letters was split in the middle, and if the desired letter was in the remaining group, he would again have to create a slow brain potential, and so on. Hans-Peter mostly imagined sports-related actions to control his brain potentials. Later, he even learned to turn on the brain–machine interface independently, by reacting to its background ticking sound with a specific brain potential.

In this way, it was possible to hold conversations with Hans-Peter, and, after a certain amount of practice, he was even able to write letters. Contact with my collaborator, Andrea Kübler, who visited him every day, was of vital importance for this success. Furthermore, he had the same carers looking after him for many years, who were therefore able immediately to recognise and deal with any difficult situations, such as mucus blockage in his windpipe.

Naturally, Hans-Peter’s story became a sensation. He was the first person to learn to communicate directly using the patterns of activity in his brain. His achievements were reported and honoured in the scientific journal Nature.

Locked-in patient Hans-Peter Salzmann training on the BMI

Above is the world’s first letter written using the electrical activity of the brain. In it, the patient invites the author and his collaborators to a party to celebrate his achievements.

Living wills and the power of fear

At that time, we were still caring for our patients in their homes. Today, most are cared for in the well-equipped ‘Haus CERES’ centre, near Tübingen. Over time, a friendly and trusting relationship usually develops between us and our patients. This was true of Hans-Peter. One day, he told me about his living will, which was still in his old desk drawer, and I asked him if he was happy that no one had found it back when his condition deteriorated. His reply: ‘Even judges sometimes get it wrong.’ He clearly hadn’t lost the ability to laugh at himself.

The end came while Andrea Kübler and I were away and therefore unable to care for him personally. In fact, his condition seemed stable enough not to be a cause for great concern. But our patient contracted pneumonia and was taken to a general hospital, where he was treated as a half-dead coma patient in whom they did not see any point in investing anything, either in terms of medical treatments, or in terms of care. Hans-Peter developed bed sores and died within two weeks, without having exchanged a single word more with anyone.

Hans-Peter’s death is all too typical and certainly no rarity, although there is no statistical information on this issue. Young doctors in particular are often unable to recognise that a totally paralysed patient has any quality of life and — like many other people — are more inclined to think of ‘putting an end to their suffering’. The consequence of this attitude is that they avoid contact and interaction with such patients. Their lack of training in dealing with such patients means they are not able to examine them objectively. Dangerous infections and breathing difficulties are ignored, and this is true not only of most doctors, but most nursing staff, too. What both professions have in common is that they are trained to cure patients, even though modern medicine is increasingly about treating people with chronic, incurable conditions, whom medical staff should not be aiming to cure, but to provide with as high a quality of life as possible despite their illness.

Hans-Peter’s death was as unnecessary as it was tragic. It would not happen under our care now, as we now make sure that we are informed of any changes to our patients’ state of health. But after Hans-Peter died, our only comfort was that he had at least had eight years of worthwhile life after he required artificial ventilation, which he would not have had if the wishes expressed in his living will had been followed.

This experience is one of the main reasons we now try to reach an agreement with our ALS patients that there should be no hasty withdrawal of life support. In fact, we try to agree on a waiting period of up to a year following the start of artificial ventilation. Patients who still wish to die after that period can rely on our help. In the case of ALS patients, this means they are given benzodiazepine and a dose of morphine to make them fall asleep peacefully. This is legal in many countries, including Germany, but is practised in only a few clinics, doctor’s practices, and care homes. Often, the ventilator machine is simply switched off or artificial feeding is stopped, which ultimately means that they die a wretched death from suffocation or starvation. This can only be described as inhumane and brutal, irrespective of the condition the patient is in. Nonetheless, many people give their express permission for this in their living will, as Hans-Peter originally did. This presumably means they did not properly understand the document they signed.

This once more makes clear that the problem with living wills is that they are usually signed without the requisite knowledge. At the moment of signing, a signatory to a living will cannot know how he or she will feel at the time the will comes into force. Just as he or she cannot know how it feels to starve to death or suffocate when totally paralysed. People allow themselves to be taken in by the idea that rejecting all life-support measures in advance is a free and independent decision on their part. This is a fallacy of breathtaking proportions, a confusion of limitless freedom with limitless stupidity. Freedom to decide can only come if we know what the options are — in this context, what the alternative to switching off the machines is. But that is not the case by far!

Most of us have probably heard about patients left paralysed by a stroke and perhaps even seen with our own eyes how they require constant care and have no control over their own body. But who knows what’s really going on inside their heads? And has anyone ever seriously considered the possibility that such people might actually be happy and feel that their lives are fulfilling? There is a huge gap in people’s knowledge here, which each individual should seek to redress for him- or herself. However, most people prefer to go with their spontaneous fears and sign a living will, which may turn out to be more of a curse than a blessing in the future.

Very few people, both within families and within the medical profession, are aware that it is possible to communicate with totally paralysed patients via the BMI. Despite this lack of knowledge, health-insurance companies have never refused to cover the cost of this procedure in any cases that I know of. Those costs amount to about €30,000 at the time of writing, but they are falling every year. There is much industrial research, particularly in Germany, aimed at producing affordable, wireless BMI devices.

Unfortunately, my institution is the only one to have staff trained in BMI procedures, a state of affairs that must be addressed urgently. This brings me to the crux of the matter. Any progress in the field of BMI ultimately depends on whether doctors and family members want locked-in patients to communicate with them and to return to the land of the living. As long as the prevailing attitude is that it is better to ‘switch off’ such patients, to ‘put them out of their misery’, progress in developing BMI technology and techniques, as well as efforts to lower the costs, will continue to stagnate and may even come to a complete halt. And then it will be no use looking abroad for help.

More relaxed laws cause more fear

In countries such as Belgium, the Netherlands, and Switzerland, and in some US states, pseudo-liberal legislation has meant that euthanasia has become practically routine. It is sometimes even requested in cases of depression. Some would like to see similar regulations in Germany. But I can only warn against this, because I believe we are justified in our suspicion that relaxing euthanasia laws simply reinforces patients’ negative expectations when it comes to their future quality of life. The message they receive is that if everyone is calling for legalised euthanasia, the alternative — for example, life with advanced Alzheimer’s or locked-in syndrome — can only be terrible.

In Germany, by contrast, where reaction against the lasting legacy of the policies of the Nazis means legislative attitudes to euthanasia are relatively restricted, such patients do not have such a negative view of their future at all. When we spoke to ALS patients about the issue of assisted dying, only three out of 100 respondents said they had seriously considered it and had already broached the subject with their doctor. Clearly, their fear of a miserably low quality of life in the future had been so great as to make them want to put an early end to their life. Yet even those three had decided against it in the end.

The decisions people make are often driven more by fear than by rational insight. As Nietzsche put it, ‘fear is the mother of morality’. In Nietzsche’s time, there was no such thing as a living will, and, even just 20 years ago, barely anyone was keen to sign one, because the fear of death was greater than the fear of suffering or of losing control of life and limb. The main thing was to stay alive. This is not the case today — even in Germany, where the movement for the relaxation of euthanasia laws is making itself ever more keenly felt.

In January 2013, the German health insurer DVK published the results of a representative survey in which almost 60 per cent of 45-year-olds said they would rather be dead than suffer dementia. It seems reasonable to assume that a similar proportion would give the same answer in the case of locked-in syndrome, which is also tied up with the fear of losing independence and self-determination. Living-will and euthanasia legislation has nothing to do with moral progress or increasing personal responsibility, and certainly nothing to do with an increase in knowledge; it is nothing more than an irrational displacement of people’s fears. And this attitude feeds off the fact that we massively underestimate the brain’s resources and its plasticity — and therefore its abilities to heal itself.